General
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• On chromosome 1q22-23 (not MHC linked); non-covalently associated with beta2 microglobulin
• Capable of binding ligands with greatly varying alkyl chain lengths (including Mycobacteria tuberculosis and Mycobacteria leprae) through a complex network of interconnected hydrophobic pockets (J Immunol 2004;172:2382, Proc Natl Acad Sci USA 2011;108:19335, OMIM #188360)
• Part of immune response to intracellular bacteria
• Human gammadelta T cells recognize Lipid A in a CD1b or CD1c restricted manner as a first response to gram negative bacteria (Biol Direct 2009;4:47)

Diagrams
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MHC class I-related lipid-binding proteins

Uses by pathologists
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• No significant clinical use by pathologists

Micro images
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Leprosy patient

Thymus tissue

Urinary bladder shows strong cytoplasmic
positivity in urothelial cells

Positive staining - normal
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• Cortical thymocytes, Langerhans cells (weaker staining than CD1a), myeloid dendritic cells, brain pyramidal cells and subpopulation of B cells

Positive staining - disease
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• Dendritic cells in mycosis fungoides (J Cutan Pathol 1995;22:223), myeloid leukemia, some B and T cell malignancies
• Monocytes of most sickle cell anemia patients (Hum Immunol 2004;65:1370)

Negative staining
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• Normal B cells

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